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Titolo:
NEONATAL INTRAHEPATIC CHOLESTASIS WITH HEPATIC SIDEROSIS AND STEATOSIS
Autore:
TAZAWA Y; NISHINOMIYA F; ABUKAWA D; AIKAWA J; OHURA T; TOHMA M; WATANABE A; SUZUKI T; TAKADA G; KONNO T;
Indirizzi:
TOTTORI UNIV,FAC MED,DEPT PEDIAT,36-1 NISHIMACHI YONAGO TOTTORI 683 JAPAN AKITA UNIV,SCH MED,DEPT PEDIAT AKITA 010 JAPAN TOHOKU UNIV,SCH MED,DEPT PEDIAT SENDAI MIYAGI 980 JAPAN HLTH SCI UNIV HOKKAIDO,FAC PHARMACEUT SCI HOKKAIDO JAPAN TOHOKU UNIV,INST DEV AGING & CANC,DEPT PEDIAT ONCOL SENDAI MIYAGI JAPAN
Titolo Testata:
Acta Paediatrica Japonica Overseas Edition
fascicolo: 2, volume: 40, anno: 1998,
pagine: 150 - 154
SICI:
0374-5600(1998)40:2<150:NICWHS>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
IRON; HEMOCHROMATOSIS; LIVER; DEFICIENCY; PROFILES; ENTITY; ACIDS;
Keywords:
FATTY LIVER; HEPATIC SIDEROSIS; HYPERFERRITINEMIA; NEONATAL INTRAHEPATIC CHOLESTASIS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
29
Recensione:
Indirizzi per estratti:
Citazione:
Y. Tazawa et al., "NEONATAL INTRAHEPATIC CHOLESTASIS WITH HEPATIC SIDEROSIS AND STEATOSIS", Acta Paediatrica Japonica Overseas Edition, 40(2), 1998, pp. 150-154

Abstract

Neonatal intrahepatic cholestasis is a heterogeneous disease of undetermined cause. There is an unreported subset of idiopathic neonatal intrahepatic cholestasis with an unusual histological combination of hepatic siderosis and macrovesicular steatosis. The patients were a 34-day-old female and a 39-day-old male with normal birth weights. Their mothers had received oral iron supplement 4-6 weeks before delivery. Thepatients had obstructive jaundice noticed at the well-baby clinic at I month of life. They had high levels of serum galactose and tyrosine,hyperferritinemia. Urinary organic acid and bile acid analyses were negative, and galactose-1-phosphate uridyltransferase activity in red cells was normal. Liver biopsies showed diffuse iron deposits and macrovesicular fat. By substituting formula milk with lactose-free milk, the patients responded, and had normal biochemical tests within 5 monthsof life. Follow-up biopsies, at the age of 12 months, showed mild residual fibrosis without iron or fat deposits. They are both well at 3 and 6 years of age, respectively, without biochemical liver dysfunctionand neurologic impairment. Prenatal iron-overload might contribute tothe pathogenesis of the disease, but further studies are needed to confirm the assumption.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/09/20 alle ore 22:06:33